Hornik Christoph P, Collins Ronnie Thomas, Jaquiss Robert D B, Jacobs Jeffrey P, Jacobs Marshall L, Pasquali Sara K, Wallace Amelia S, Hill Kevin D
Department of Pediatrics, Duke University, Durham, NC; Duke Clinical Research Institute, Duke University, Durham, NC.
Division of Pediatric Cardiology, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, Ark.
J Thorac Cardiovasc Surg. 2015 Jun;149(6):1516-22.e1. doi: 10.1016/j.jtcvs.2015.02.016. Epub 2015 Feb 14.
Patients with Williams syndrome (WS) undergoing cardiac surgery are at risk for major adverse cardiac events (MACE). Prevalence and risk factors for such events have not been well described. We sought to define frequency and risk of MACE in patients with WS using a multicenter clinical registry.
We identified cardiac operations performed in patients with WS using the Society of Thoracic Surgeons Congenital Heart Surgery Database (2000-2012). Operations were divided into 4 groups: isolated supravalvular aortic stenosis, complex left ventricular outflow tract (LVOT), isolated right ventricular outflow tract (RVOT), and combined LVOT/RVOT procedures. The proportion of patients with MACE (in-hospital mortality, cardiac arrest, or postoperative mechanical circulatory support) was described and the association with preoperative factors was examined.
Of 447 index operations (87 centers), median (interquartile range) age and weight at surgery were 2.4 years (0.6-7.4 years) and 10.6 kg (6.5-21.5 kg), respectively. Mortality occurred in 20 patients (5%). MACE occurred in 41 patients (9%), most commonly after combined LVOT/RVOT (18 out of 87; 21%) and complex LVOT (12 out of 131; 9%) procedures, but not after isolated RVOT procedures. Odds of MACE decreased with age (odds ratio [OR], 0.99; 95% confidence interval [CI], 0.98-0.99), weight (OR, 0.97; 95% CI, 0.93-0.99), but increased in the presence of any preoperative risk factor (OR, 2.08; 95% CI, 1.06-4.00), and in procedures involving coronary artery repair (OR, 5.37; 95% CI, 2.05-14.06).
In this multicenter analysis, MACE occurred in 9% of patients with WS undergoing cardiac surgery. Demographic and operative characteristics were associated with risk. Further study is needed to elucidate mechanisms of MACE in this high-risk population.
接受心脏手术的威廉姆斯综合征（WS）患者面临重大不良心脏事件（MACE）风险。此类事件的发生率和风险因素尚未得到充分描述。我们试图通过多中心临床登记来确定WS患者中MACE的发生频率和风险。
我们利用胸外科医师协会先天性心脏病手术数据库（2000 - 2012年）确定WS患者所进行的心脏手术。手术分为4组：孤立性主动脉瓣上狭窄、复杂左心室流出道（LVOT）、孤立性右心室流出道（RVOT）以及联合LVOT/RVOT手术。描述发生MACE（院内死亡、心脏骤停或术后机械循环支持）的患者比例，并检查其与术前因素的关联。
在447例初次手术（87个中心）中，手术时的年龄中位数（四分位间距）和体重分别为2.4岁（0.6 - 7.4岁）和10.6千克（6.5 - 21.5千克）。20例患者（5%）死亡。41例患者（9%）发生MACE，最常见于联合LVOT/RVOT手术（87例中有18例；21%）和复杂LVOT手术（131例中有12例；9%）后，但孤立性RVOT手术后未发生。MACE的发生几率随年龄降低（优势比[OR]，0.99；95%置信区间[CI]，0.98 - 0.99）、体重降低（OR，0.97；95% CI，0.93 - 0.99），但在存在任何术前风险因素时增加（OR，2.08；95% CI，1.06 - 4.00），在涉及冠状动脉修复的手术中增加（OR，5.37；95% CI，2.05 - 14.06）。
在这项多中心分析中，接受心脏手术的WS患者中有9%发生MACE。人口统计学和手术特征与风险相关。需要进一步研究以阐明这一高危人群中MACE的机制。
